[Management of muscle cramp: what's to be done?]. / Comment optimiser la prise en charge des crampes musculaires?

Muscle cramp is characterized by involuntary, painful, visible contraction of a muscle (or a part of muscle) and is always associated with irregular repetitive firing of motor unit action potentials (200 à 300 Hz) which is caused by hyperexcitability of intramuscular terminal motor axons. It's a common condition in normal people, but most commonly in young people (pregnancy, exercise) and more in the elderly (50% after 65 years-old). A careful history and examination should allow the physician to determine the significance of cramp. ENMG and biological tests are needed in cases of severe symptoms (severity and frequency of cramps) and/or abnormal examination. Idiopathic and secondary (drug or metabolic disorders) cramps are the most common groups, but it's very important to search the motor unit diseases (neuropathy, radiculopathy, plexopathy, neuromyotonia, and a cramp fasciculation syndrome which can preceded ALS). The first goal in management of cramp is to determine if there is an underlying cause and the second to use physical measures (stretching), because, pharmacologic treatments have a moderate interest because of the potential of toxicity (quinine sulfate) or a little effectiveness (vitamin B complex, naftidrofuryl, and calcium channel blockers such as diltiazem, gabapentin). Isolated cramp doesn't need treatment.

At-risk phenotype of neurofibromatose-1 patients: a multicentre case-control study.

OBJECTIVES: To assess associations between subcutaneous neurofibromas (SC-NFs) and internal neurofibromas in patients with neurofibromatosis type 1 (NF-1) and to determine whether the association between SC-NFs and peripheral neuropathy was ascribable to internal neurofibromas. PATIENTS AND METHODS: Prospective multicentre case-control study. Between 2005 and 2008, 110 NF-1 adults having two or more SC-NFs were individually matched for age, sex and hospital with 110 controls who had no SC-NF. Patients underwent standardized MRI of the spinal cord, nerve roots and sciatic nerves and an electrophysiological study. Analyses used adjusted multinomial logistic regression (ORa) to estimate the risk of the presence of internal neurofibromas or peripheral neuropathies associated with patients presented 2 to 9 SC-NFs, at least 10 SC-NFs as compared to patients without any (referential category). RESULTS: Cases had a mean age of 41 (± 13) years; 85 (80%) had two to nine SC-NFs and 21 (19%) at least ten SC-NFs. SC-NFs were more strongly associated with internal neurofibromas in patients with ten or more SC-NFs than in patients with fewer NF-SCs (e.g., sciatic nerve, aOR = 29.1 [8.5 to 100] vs. 4.3 [2.1 to 9.0]). The association with SC-NFs was stronger for diffuse, intradural, and > 3 cm internal neurofibromas than with other internal neurofibromas. Axonal neuropathy with slowed conduction velocities (SCV) was more strongly associated with having at least ten SC-NFs (aOR = 29.9, 5.5 to 162.3) than with having fewer SC-NFs (aOR = 4.4, 0.9 to 22.0). Bivariate analyses showed that the association between axonal neuropathy with SCV and sciatic neurofibromas was mediated by the association between SC-NFs and sciatic neurofibromas. CONCLUSION: The at-risk phenotype of NF-1 patients (i.e. NF-1 patients with SC-NFs) is ascribable to associations linking SC-NFs to internal neurofibromas at risk for malignant transformation and to axonal neuropathies with slowed conduction velocities. Axonal neuropathies with SCV are particularly common in patients with at least ten SC-NFs.

[Meningitis due to Capnocytophaga canimorsus without dog bite]. / Méningite à Capnocytophaga canimorsus sans notion de morsure de chien.

INTRODUCTION: Capnocytophaga canimorsus is a fusiform and filamentous gram-negative rod, part of the normal oral flora of dogs and cause rare human febrile acute meningitis, usually severe but curable. OBSERVATION: A sixty years old man presented a severe acute meningitis with fever and confusion. CSF show 4,000 cells/mm3 with 83% neutrophilis, increased protein level (5,02 g/L), very low glucose and positive Gram stain result. The patient fully and quickly recovered with antibiotherapy for 22 days. Bacteriological diagnosis was made by genomic study from CSF culture. The patient has a close contact with his dog without being recently bitten. DISCUSSION: Diagnosis, suggested by bites or contact with dog or cat, gram-negative bacilli with gram stain of CSF specimen, is possible by prolonged culture of CSF or blood sample, with if necessary genomic study. Antibioprophylaxis is strongly recommended in cases of deep bite wounds and for immunocompromised patients.

Neurofibromatosis 1-associated neuropathies: a reappraisal.

Neurofibromatosis 1 (NF1) is a common disease which is a source of various multisystemic manifestations related either to the accumulation of neurofibromas or to specific developmental abnormalities. The neurofibroma is the hallmark lesion of NF1 and develops from peripheral nerves. However, to date, the description of peripheral neuropathies of NF1 has not been investigated. To examine this question, we have evaluated 688 NF1 patients for the presentation, prognosis and associated morbidity of peripheral neuropathies in two hospital-based series. We collected 18 patients (four women and 14 men) with diffuse peripheral neuropathy (2.3%). Eight patients had a paucisymptomatic or an asymptomatic neuropathy detected only on electrophysiological study, two had minor sensory manifestations, five had moderate motor and sensory manifestations and three had severe motor and sensory manifestations. Superimposed radicular changes were observed in seven cases. Two patients had a subacute and 16 a chronic polyneuropathy. Fourteen patients had a demyelinating neuropathy with either severe axonal changes (three), moderate or minor axonal changes (four) or no axonal changes (seven). Four patients had axonal neuropathies. There was a strong association between the presence of a peripheral neuropathy and large root diffuse neurofibromas (P < 0.03) and subcutaneous neurofibromas (P < 0.0001). Severe morbidity and mortality of patients with NF1 and peripheral neuropathies was 50%, much higher than what is observed in the general population of patients with NF1, and 100% in patients with the most severe symptoms and electrophysiological changes (demyelination with severe axonal features). Four patients out of 18 (22%) developed a malignant peripheral nerve sheath tumour (MPNST), a much higher proportion than in the whole population of NF1. Two patients died. Peripheral neuropathy constitutes a potentially severe complication in patients with NF1 associated with a frequent morbidity related to spinal complications and MPNSTs. Association of proximal large neurofibromas, peripheral neuropathies and subcutaneous neurofibromas may constitute a phenotype of NF1 with a severe prognosis.